Wednesday, August 31, 2011

We had a routine clinic visit on Monday morning and it went pretty well. We're still waiting on the results of his culture and are hoping it comes back clean. Two other things that came from the appointment was scheduling a hi-res CT scan and a discussion about whether to start on Azithromycin three days a week.

First, the CT. We had talked about it with our doctors before and decided that now was a good a time as any to get it done. Our clinic has started to talk more about this procedure recently, as some studies have shown that a hi-res CT scan can show bronchiectasis early on better than any other test and medicines and therapies can be adjusted to try to better clear the airways preventing infection and further bronchiectasis. Bronchoscopy is good at looking at mucus plugging and other airway abnormalities, but can't see into the smaller, lower airways where lung damage starts. The hi-res CT is useful over time to see how lung disease progresses. I hate talking about lung disease in my 1 year old, but with Cystic Fibrosis, its just the reality. Anyway, his doctor said that she suspects that they will see some lung disease just because of his inability to appropriately clear secretions. She said that some kids have it at this age and some don't, but if she had to guess, she would suspect that he has some damage. The procedure is non-invasive, but he does have to get put under for it. We have it scheduled for the end of the month. I won't worry about it until a little later on :)

The other thing that we discussed at this clinic visit was putting Drew on regular Azithromycin. Azithromycin is an antibiotic (you may know it as a z-pak) used to treat common bacterial infections. But it wouldn't be used as an antibiotic in Drew's case. Here's some information about this from the CFF website:

Azithromycin is a commonly used antibiotic made by Pfizer, Inc. and sold as Zithromax®. Many people have taken this drug to treat pneumonia, sore throats or ear infections.

In 1999, the Cystic Fibrosis Foundation recognized that azithromycin might help people with cystic fibrosis (CF) who have a positive sputum culture for Pseudomonas aeruginosa(Pseudomonas) and designed a clinical trial to test that possibility.

During the first clinical trial, lung function (FEV1), weight, use of antibiotics, and days spent in the hospital to treat lung infections were watched for changes. The people with CF who were involved in the study were split into two groups. One group took a placebo—an inactive pill that has no treatment value (sometimes called a sugar pill). The other group took azithromycin.

During this trial, neither the patients nor the CF researchers knew which patients took azithromycin or placebo pills. Over six months, the group that took the azithromycin had about a 6 percent improvement in their lung function, fewer pulmonary exacerbations, took fewer antibiotics (other than azithromycin) and had an increase in their weight. They also spent 47 percent fewer days in the hospital for the treatment of a lung infection.

In the second study, the kids with CF did not have Pseudomonas for at least one year, were 6 to 18 years of age, and had mild to moderate lung disease, although most had mild lung disease.

Over six months, the group that took the azithromycin had fewer exacerbations, took fewer oral antibiotics and had an increase in their weight.

Lung function was about the same in those who took placebo and those who took azithromycin. However, the people in the azithromycin group reported less coughing than the group taking the placebo.

That sounds like reasonably good evidence for me to get him on Azithromycin. My only hesitation is that it then takes Azithromycin out of the running as an antibiotic when he does get a lung infection, and Azithromycin is something that we've had reasonable success with when we needed to use it in the past. If Drew would be on Azithromycin 3 days a week ongoing, his body will build up a resistance to its antibiotic effects, thus eliminating it as an effective treatment option for us. There are plenty of other antibiotics that he can get put on if he gets sick, but hes on so many antibiotics all the time that there is always a fear of his body building up resistance and us not having the options we need to treat his infections.

We decided to see what we see in the CT and then make a decision on the Azithromycin. If he does have reasonable lung disease and bronchiectasis then we will more likely than not go ahead and get him on it. Maybe if he looks good too, I'm just not sure yet.

Friday, August 26, 2011

Today was the big day and it went awesome! At the last minute, I got a call from the Congresswoman's district scheduler and something had come up with the Congresswoman and she wasn't going to be able to make it to our meeting, but I was given the option of meeting with her staff. Definitely not the option I wanted, but it was a foot in the door, and it turned out to be spectacular!

I met Lisa Burns (Drew's doc) about 15 minutes beforehand to briefly go over what I wanted to discuss. I had told her that I was nervous. I like to think of myself as well educated and knowledgeable in many areas, however government is not one of those areas. When I had been discussing this with Martin, he told me to just keep in mind that she works for us, that we are her constituents and she represents us in Congress. Good point Martin Moore, good point. So I went in and we met with the Deputy District Director named Sara. I told her a little bit about Drew and our CF journey before going into the nuts and bolts of why I was there. She was a really nice lady who seemed very interested in what we were talking about, asking lots of questions and seeming to genuinely care about our discussion. She mentioned that she hadn't know much about CF until recently when a close friend had a baby who was born with it. It was as if the meeting with the Congresswoman was supposed to fall through so we could meet with this lady. I mean couldn't have worked out more perfectly.

I told her that Cystic Fibrosis isn't federally funded, and that I wasn't there asking for their support to get funding (though should they choose to, I'd be okay with that). What I wanted was support in getting the treatments through the FDA for approval, and the way she could help was to support budget cuts that DO NOT affect the FDA. Additionally, on matters concerning the NIH (National Institute of Health), support funding and oppose all cuts, as budget cuts to the NIH could cause the life saving research that's going on in places like Cincinnati to come to a halt. There is a group in Congress know as the Congressional CF Caucus and I said that if I had 1 action item for the Congresswoman it would be to ask for her to join this group. This group of representatives fights for the things listed above, and to have Congresswoman Schmidt as a part of that group would be huge. A large portion of her Congressional District utilized the services offered by Cincinnati Children's Hospital, one of the leading CF research hospitals in the country. And I'm sure that a fair number of the employees of the hospital live within her district as well. To have her fighting for a cure with us would be huge, and I'll write and educate and inform until we get there.

It really was a great meeting and I'm glad that I was able to set it up and go through with it. I was definitely nervous going in, but after the meeting, I realized that if I'm not fighting for Drew's life, no one will. I need to be his voice and I need to fight for what he needs, for what we need. I very briefly touched on Medicaid, simply noting that my husband has a great job and we have great insurance, but without our secondary insurance for Drew, Medicaid, we would be paying something like $74,000 a year, just for medications!!! Actually, we probably wouldn't be because we don't have an extra $74,000 laying around (I know, surprising) and what would happen is he would have to go without treatments and his health would deteriorate. And my heart would break at the same time. I asked for her support in preserving programs like Ohio's Bureau for Children with Medical Handicaps (Medicaid) for children and adults with CF so that they can receive the treatments they need to live the life they deserve.

I left the meeting and let Sara know that I would still love the opportunity to meet with Congresswoman Schmidt, and hopefully that request will come to fruition at some point. But for now I'm happy with what I accomplished.

Thursday, August 25, 2011

Tomorrow is the big day that I'll be meeting with our local Congresswoman to ask for her support. I've been practicing what I'm going to say about Drew, what I'm going to ask for, and what I want her to know and remember. I'm definitely a little anxious, but I just have to keep in mind that I know more about CF than she does, and I'm just there to tell my story and ask for her to support the policy issues affecting CF - stuff I do all the time on this blog and via emails and FB posts. I'll let you know how it goes!

In other great news, my sister called today to let me know that she got a new job as Development Manager for the Greater Philadelphia Cystic Fibrosis Foundation! She wasn't even really looking, but had just become friendly with some folks from the Foundation through her volunteering and when the position opened up they offered it to her! There was some interviewing involved, but at the end of the day it all worked out just the way it was supposed to. She's been interested in event planning since college, but like most 2005 grads, was left to deal with the wonderful state of the economy and taking whatever jobs they could get. She starts in September and will be responsible for managing several of the Philadelphia area walks next year. Go Kate!

I got a little more info on the NACFC and desperately want to go, but just don't think its in the cards this year. Had we been able to plan for it, it might have worked it, but its coming up quickly (Nov.3-5) and its going to cost a lot. I might just have to weasle my way into getting the handouts and presentations from one of the docs or nurses in our clinic who will be attending. I'm not totally giving up hope yet. If I magically fall upon airline tickets and a hotel willing to put me up for 3 nights free of charge, I'm so there ;)

Monday, August 22, 2011

There was a bit on the Today show this morning about toxic friends. The statistic that they gave on the show was that 84% of women have at least 1 friend who makes life difficult. I can buy that. They said that the friendship that is causing you stress can have a negative impact on your health and well being. They broke these "toxic friends" into several categories - The Narcissist, The Chronic Downer, The Critic, The Underminer, and The Flake. I think we can all say that we've at least known, if not been friends with, a person who fits into at least one of these categories, and its hard to be friends with them. So I'm breaking up. I am walking away from these toxic friendships. I pick my friends because they are people that I enjoy, that make me feel good about myself. When I hang out with my friends, I want to leave feeling happy and satisfied, not come home and bitch about them and why they make me crazy. I've got bigger things in my life to worry about than The Underminer, The Critic and The Narcissist.

This brings me to odds and ends #2. Let me first say that I, too, am guilty of this, and I'd like to change it about myself. It makes me nuts when people pass judgement on the way other people parent. I know we all think we're doing it the right way. But whose business is it to say how much time you should or shouldn't be spending with your kids and how much TV they should or shouldn't watch and where you should or shouldn't send them to school. If spending every minute of every day with my children is what makes me happy, then that's what I'm gonna do. They grow up so fast and I don't wanna miss a thing. This was a big thing for me when we had the twins and Drew was in the hospital, and its come up again lately since I started volunteering at Children's. Its so easy for people to tell you what you're supposed to do - that you need to spend more time with your husband or get out of the house a little more often or whatever. And what I've shared with the folks in the NICU that I talk to is this - its you life, live it as you see fit. For some people, spending time with their significant other is the most important thing in the world to them. For other, myself included, its tucking my kids in at night with a hug and a kiss and a promise of a good day tomorrow. We almost never go out before the kids go to bed. Its what's important to us. If you choose differently, that's fine, but your decision is no better than mine.

I just needed to rant for a few minutes, now I'll get into the CF news I've got. First things first, I've got a meeting this Friday with our local Congresswoman to ask her to participate in the Congressional CF Caucus and to support funding for the FDA and the NIH in FY 2012 to keep the programs that are so vital to people living with Cystic Fibrosis. And the even better news is that the totally awesome Dr. Lisa Burns will be coming with me! I'm not exactly what I'd call savvy when it comes to matters of government. I know what I know and what I believe in and support, but that's about the breadth of my knowledge. What I think really needs to happen at this meeting is simply for Congresswoman Schmidt to hear our story and understand that Cincinnati Childrens Hospital, within her congressional district, is one of the top CF research facilities in the country, and that's what her support of our cause is crucial as we head into some big things in the coming years. I'll let you know how that goes after the meeting.

In other big news, Dr. Burns told me that a doctor named Colby Wyatt, who just so happened to be a resident in the NICU when Drew was in there and took care of Drew for about a month when he was first born, decided to do his fellowship in Pulmonary. Dr. Burns and some other encouraged Dr. Wyatt to submit Drew's case for the North American CF Conference as the Fellow's Presentation and it was accepted!! Drew is going to be the case study discussed at the conference in November! Its the largest meeting of CF doctors and caregivers in the country and they're all going to be talking about Drew! I think that's incredible and I really, really want to go. Its in Anaheim, CA November 3-5. I made a few calls today and it looks like with the approval of the director of your CF Clinic, patients and parents can attend. Its directed toward medical professionals, but I don't really care. Knowledge is power, baby. I'm sure I'd have no problem getting the director of our clinic to sign off on that, as Dr. Burns suggested that I would probably really enjoy it and I know she'd support me if I needed her so. There is a registration fee that isn't totally unreasonable, but then there is airfare and hotel and a rental car and the cost just seems to add up and might just be more than we can afford right now. We're going to talk about it this weekend and see if we can make it work. In the meantime, if anyone reading this cares to sponsor me, i'm open to it :)

Wednesday, August 17, 2011

I've been absent from the blog for the past week or so. We are on vacation in Charleston and I spent last week packing and planning and this week enjoying the gorgeous weather, the beach, and the company. We got down here a lot quicker than we expected which was great. We still spent a night in a hotel just to get the kids out of the car and have a place to eat and get cleaned up before we could check into our house. And the house - there are 19 of us (not 22 as previously stated). There are 10 bedrooms and 7 or 8 bathrooms. The pool is almost always full of kids and adults and there is enough food here to feed a small army. Well, 19 of us anyway.

On Thursday of last week, the day before we left, Drew was doing a lot of coughing. He had just come off Azithromyacin and I thought he was getting better, but I started to get nervous with all the coughing that was going on. I got him steroids and, per the usual, they did the trick. There was one day down here, Monday or Tuesday, that he was sounding a little junky but we wrapped up the steroids last night and he's been sounding good. I feel like with 19 people in the house, germs are bound to spread. I just hope that we can make it through the rest of the week without incident and remain healthy for a couple more weeks before Ella starts preschool.

I'm not gonna lie, I thought the malacia would be better by this point in Drew's life. And maybe it is. Maybe it's the airway abnormalities that still cause him extra difficulty and inflammation when he gets sick. Or maybe this is just what CF is for us. I feel like we just got through winter and it's creeping back up on us again already. I'm hellbent on staying out of the hospital unless absolutely positively necessary. I guess we go in with a positive attitude and just play the hand we're dealt.

Our next clinic visit is at the end of the month. This is the longest we've gone without a visit. It's been two months since we've had a culture and had to wait and wonder what it was going to come back with. I think our new doctor will be credentialed by then and we will see her from now on. She's awesome (loved our old doc too, and frankly almost everyone we've worked with at the clinic) and it will be nice settling into just one doctor and nurse.

The small ones have all been put down for the night and it's game time for the grownups. Three more nights on vaca before we get back to the grind.

Monday, August 8, 2011

Well, two of the 5 of us are on antibiotics. I got bronchitis last week and I'm still hacking my brains out. Drew started sounding kind of junky last week, maybe around Wednesday. I called the doctor on Friday when he was sounding progressively worse. He wasn't having any trouble breathing, just lots and lots of coughing, and a gunky nose. I know that not all colds that CF kids get turn into bacterial infections, but with Drew's malacia, more often than not, when he's got extra mucus in his airways it starts to grow bacteria. Plus, with me having bronchitis and being on antibiotics, I just didn't really see Drew getting better on his own.

The other factor weighing on my decision to call the doctor and request antibiotics was that we're leaving for vacation on Friday. I don't want to drive to Charleston, SC and end up doing airway clearance 4 times a day and having Drew struggling to breathe and miserable. We got him on Azithromyacin Saturday morning and he started sounding better by Sunday. He's still a little junky sounding. We're added hypertonic saline once more per day and are using the vest 3 or 4 times (depending on our schedule). He's still needing Atrovent a little bit more than he normally does but I think once he gets some of this cleared we will get back to normal.

I have to say that over the past several months, there's been a dramatic and noticeable improvement in the sound of his breathing (read: I think his malacia is getting better). It will be nice when you don't hear him breathing all the time. We're use to it, but people who don't know him look at us like he's going to croak. Lucky for Drew, i'm not one to keep my words to myself and quickly quiet their chatter about how sick that poor kid must be. If they only knew.

I haven't started packing yet for our trip. We're driving. I'm not looking forward to that, but I'm not really to worried about it either. When we went to Philadelphia a few months ago, we only stopped twice on the way out and once on the way back, making the 8 hour trip in under 9 hours. Google tells me it will take about 10-11 hours to get to Charleston. We're planning to get up a little early, get all Drew's breathing treatments done, and head out by about 8am. We have a hotel booked but we're not sure if we're going to use it or push through. We can't check into our house until Saturday, but Martin has a brother who lives in Charleston that we might crash with. We'll see how the trip is going. I would love to do it all in one day only because then we don't have to spend any time in the car the next day. I guess we'll see.

It should be a pretty fun vacation. There are 22 of us staying in a giant 10 bedroom house a block from the beach. We have a pool too. We're going with Martin's whole family. Ella will have a blast playing with her cousins and it will be nice to hang out with his parents & siblings and their spouses too. Mostly, it will be a nice break from the everyday here in Cincinnati. I love these kids dearly and I wouldn't change my job for the world, but its nice to have a break in your routine. And I've heard that being near the salty ocean with the salty ocean air will be like giving Drew his Hypertonic Saline 24/7 and hopefully improve his breathing. Maybe this could work out to be an argument for getting a beach house :)

Wednesday, August 3, 2011

Several years ago, we got together with friends of ours and had an Oktoberfest party. The guys made their own sausage (yes, ground it, seasoned it, stuffed it, grilled it, ate it), and beer, and the girls made the sides and organized the friends. We had the party for 3 or 4 years I think, and then last year with all the drama of twins, had to cancel it. This year, we've decided to revive the party, but we've turned it into a fundraiser! For $25 per couple, you get all the food you can eat and beer you can drink. Or if you don't want to pay, you can donate an item worth at least $50 to be used at an upcoming CF event in their silent auction. I think its a pretty good deal, really. We're hoping for at least 50 people to attend this year, but even more, we're hoping to perfect this and make it our huge yearly fundraiser. Here is the link to the invite - everyone is welcome. You can purchase tickets online in advance by clicking on the link on the left-hand side of the blog where is says "Oktoberfest Tickets - DONATE". I hope we can make this a big success!

Tuesday, August 2, 2011

I wanted to share an email i received from the Advocacy Chair about a few articles focusing on CF that were recently in Nature Magazine, which will be shared with some congressional offices over the next several weeks during the Make Every Breath Count campaign. They discuss the Foundation’s creation of “venture philanthropy” as a successful way to prompt the development of treatments, intellectual property issues raised by venture philanthropy partnerships, and the reasons why researchers should welcome this strategy. If anyone reading this works for a biotech or pharmaceutical company, your support and consideration in participating in such a partnership would be awesome.

"Venture philanthropy” is a concept developed by the Cystic Fibrosis Foundation where a non-profit organization partners with a biotech or pharmaceutical company and contributes funds to the company’s research into disease-specific treatments. Developing rare disease drugs is a risky undertaking for a drug company, as it can be expensive and the probability of a return on investment can be low. Venture philanthropy helps to lessen this risk and has led to the development of over 30 drugs to treat cystic fibrosis. It is now widely used in the non-profit community.

The first article, entitled “Charities seek out drug royalties,” explores how the CF Foundation and other non-profits retain certain rights to the treatments they help fund and discusses a now widely-used legal strategy crafted by CFF to ensure that a therapy can continue to advance even if the original developer cannot or will not continue its work. The Foundation’s foresight in not only funding drug creation but also exercising control over the development process has led to breakthrough achievements, including VX-770, and any royalties earned by CFF are funneled directly back into the Foundation’s mission of supporting vital research and drug development.

“With strings,” a Nature Magazine editorial supporting venture philanthropy, including philanthropies’ retention of intellectual property rights, cites the CF Foundation as the pioneer in this field. According to the article, “When the Maryland-based Cystic Fibrosis Foundation invested in Californian biotechnology company Aurora Biosciences in 2000, it launched a revolution.”

I haven't had the opportunity to read these articles myself as I'm not a subscriber to Nature Magazine, but if I can get my hands on them I will certainly share.

I other news, we are heading out of town for our family vacation to Charleston, SC in just under two weeks. Naturally, we are all getting sick with some sort of head/chest cold. Ella and Lily got it first. Today I feel lousy. Drew woke up with a gunky nose and I'm certain he's heading down the same path. I'm trying to decide if I should call the doctor and see if we can get him on some antibiotic prophylactically or if I should wait him out and see if this just passes. What I really don't want to happen is to be running all over creation next Thursday before we leave and end up needing extra treatments and medications while we are gone. I really hope it just passes. I think I'll give it another day and see what happens.

I'm happy to report that the last time we were in the hospital was 10 whole months ago! My goal is to not be in the hospital at all in 2011 (or 12 or 13, etc., etc., etc.). Health this summer has been pretty good. I'm not gonna lie, I'm not looking forward to fall and the start of preschool and winter germs. It was a long cold season last year. The hope is that Drew (and Ella and Lily) have built up some immunities and won't get as sick this year. The other hope is that with the improvement in Drew's malacia, he'll have a little more success clearing secretions when he's sick. I guess time will tell.

"65 Roses"is what some children with cystic fibrosis call their disease because the words are much easier for them to pronounce.

This blog, 66 Roses, is dedicated to finding the cure.

Cystic fibrosis is a life shortening genetic disease that affects the lungs and digestive system of about 30,000 children and adults in the United States (70,000 worldwide). Thick mucus clogs the lungs and leads to life threatening infection. The pancreas is also obstructed by this thick mucus production, imparing digestion and leading to malnutrition. - www.cff.org